Outcome and Prognostic Factors of Childhood Hodgkin Disease: Experience From a Single Tertiary Center in Thailand.

In childhood, Hodgkin disease (HD) has an excellent outcome in developed countries. There are few studies on outcomes of HD from resource-limited countries. This study aimed to assess clinical outcomes and factors associated with survival rates of childhood HD in a tertiary care center in Thailand. We retrospectively reviewed the medical records of pediatric HD patients between March 1985 and August 2017. Seventy-two children diagnosed with HD were identified. Pretreatment clinical and laboratory factors were assessed by Cox regression analysis to predict event-free survival (EFS) and overall survival (OS). The overall 5-year EFS and OS rate was 70.7% and 75.5%, respectively. Multivariate analysis identified 3 factors predicting inferior EFS: high-risk group (stages III-B, IV-B), splenomegaly, and platelet count >400,000/µL. The prognostic markers were assigned a score of 1 for each factor. For a total score of 0, the 5-year EFS and OS rates were 95% and 86%; scores 2 to 3, 33% and 54%, respectively. In conclusion, our study identified 3 factors predicting inferior EFS. These adverse prognostic factors can be used in clinical practice for predicting outcomes in pediatric HD.

Survival Outcome of Alternative Medicine Treatment for Newly Diagnosed Acute Leukemia in Children.

BACKGROUND: There is scarce information on the efficacy of alternative medicine (AM) alone as a treatment for newly diagnosed acute leukemia in children. We aimed to compare overall survival (OS) between children with newly diagnosed acute leukemia who received AM alone as the first-line treatment and those treated with conventional chemotherapy (CCT). METHODS: Two-to-one nearest-neighbor propensity score-matching using sex, initial white blood cell count, phenotype of leukemia, and period of diagnosis was performed on 184 patients who received CCT and 92 who received AM alone after being diagnosed with leukemia. A multivariable Cox proportional-hazards regression model was then applied to assess the effect of treatment on OS after adjusting for potential confounders. Hazard ratios (HR) and 95% confidence intervals (CI) are provided. RESULTS: After adjusting for initial white cell count and subtype of leukemia, children treated with AM alone had worse OS (HR 5.14, 95% CI 3.75-7.04) than those given CCT. The 5-year OS rate for newly diagnosed acute leukemia treated with AM medicine alone was 0%. CONCLUSION: AM without CCT is associated with poorer survival when compared with CCT.

Clinical Course and Outcome of Childhood Acquired Platelet Dysfunction with Eosinophilia.

Acquired platelet dysfunction with eosinophilia (APDE) is a syndrome which manifests as a transient state of platelet dysfunction in the presence of eosinophilia. The aim of this work was to study the clinical course and outcomes of children diagnosed with APDE. The hospital records of children with APDE were retrospectively reviewed and a total of 69 children were included. The mean (standard deviation) age at diagnosis was 6.9 (3.1) years. All of the patients presented with ecchymoses on the extremities, body, and face. None had serious bleeding symptoms. Platelet counts were within the normal range but all of the patients had abnormal platelet morphology by light microscopy. Parasitic infestation was found in 38% and most (87%) were treated with antiparasitic drugs. The median time from the onset of symptoms to remission was 2.6 months (95% CI 1.8-3.1). The overall complete remission rates at 3, 6, and 12 months were 61, 90, and 94%, respectively, with a median follow-up time after remission of 14.0 months (interquartile range 6.0-30.8). Neither univariate nor multivariate analysis indicated any statistically significant determinants for remission time. In our study, APDE was transient with spontaneous remission, no serious bleeding manifestations, and had a benign clinical course.

Natural history of Southeast Asian Ovalocytosis during the first 3 years of life.

Southeast Asian Ovalocytosis (SAO), the most common red cell membrane disorder found in the Far-East and Pacific rim, appears to be innocuous in man since it has been identified mostly in non-anemic healthy individuals. To further substantiate our previous observation that this condition might be symptomatic particularly in the neonatal period, we studied 1567 newborns from Southern Thailand where SAO is prevalent. Thirty-one babies (1: 50 with allele frequency of 0.01) have been identified with SAO and confirmed molecularly to carry a single defective AE-1 (band 3) allele. These babies had significant anemia at birth due to hemolysis with 51.6% of them developing neonatal hyperbilirubinemia. Co-inheritance of common UGT1A1 variants in such cases was not associated with their degree of jaundice. Interestingly, hematology data of these SAO babies became "normalized" in the first 3 years of life without further evidence of on-going and/or even "compensated" hemolysis.

Cloning and characterization of pectate lyase from Hevea brasiliensis.

Latex from the commercial Hevea brasiliensis contains 30-50% (w/w) of natural rubber (cis-1,4-polyisoprene), the raw material for the many products of the rubber industry. We have constructed a cDNA library from the latex of H. brasiliensis to investigate the expressed genes and molecular events in the latex. We have isolated two cDNAs from this library, Hb-PEL-1 and Hb-PEL-2 that could encode for pectate lyase enzymes (EC4.2.2.2). From their sequence analysis Hb-PEL-1 and Hb-PEL-2 encode for proteins of 393 and 323 amino acids, respectively. Comparison of these deduced amino acid sequences with other pectate lyase enzymes showed they contained the conserved NADPH, Ca(2+) and substrate binding sites and had a 74% identity to Arabidopsis thaliana pectate lyase. Only the Hb-PEL-1 recombinant protein expressed from Escherichia coli had enzymic activity which was Ca(2+) dependent. Interestingly, Hb-PEL-1 contained an extra internal peptide between amino acid residue 38-108 when compared to Hb-PEL-2 and this peptide was also present in other pectate lyase enzymes. The transcript of pectate lyase (Hb-PEL) in the latex of rubber tree at various times after the first tapping was quantified by real-time PCR using 18s genes as internal standard. Most transcripts were detected on the first day after tapping and then decreased with time. This indicates that the pectate lyase may be involved in either the release of latex by breaking down the laticifer wall or in the development of laticifers.